Introduction
Sex cord stromal tumors (SCSTs) represent a heterogeneous group of rare
gonadal tumors that represent approximately 10% of all gonadal tumors
during childhood.1 SCSTs develop from the non-germ
cell component of the gonads. Physiologically, these cells support germ
cell maturation in their microenvironment. Furthermore, they may produce
sex hormones, and so may the corresponding tumors. The rarity and
heterogeneity of SCSTs, and the difficulty in the correct
histopathologic classification leave a significant uncertainty with
regard to the optimal clinical. Of note, some subtypes are associated
with constitutional genetic aberrations (e.g. DICER1 mutations)
and thus, they may be part of an underlying cancer predisposition and
require specific follow-up.2–4
This paper presents the internationally harmonized recommendations for
the diagnosis and treatment of children and adolescents with SCSTs,
established by the EXPeRT within the EU-funded project called PARTN-ER -
Paediatric Rare Tumours Network - European Registry. The methodology of
the process – development under the auspices of the European Reference
Network for Paediatric Cancer (ERN PaedCan) - has been already described
(Orbach et al, PBC 2021)